Volume 29, Issue 165, November 2025

Mitral valve replacement after Bentall de Bono procedure in a Patient with Marfan Syndrome – A case report

Martyna Winiarska^1♦, Dominika Wiśniewska¹, Michał Klimas¹, Dominik Jucha¹, Arkadiusz Jamro², Sabina Krupa- Nurcek³

¹Faculty of Medicine, Collegium Medicum, University of Rzeszów, Rzeszów, Poland
²Medical Center ZOZ 2 in Rzeszów, Rzeszów, Poland
³Faculty of Medicine, Collegium Medicum, University of Rzeszów, Rzeszów, Poland

^♦Corresponding author
Martyna Winiarska, Postal address: Świętej Kingi 24/44 Street, 35-614 Rzeszów, Rzeszów, Poland

ABSTRACT

Marfan syndrome is a connective tissue disorder which is caused by a mutation in the FBN1 gene, located on chromosome 15q21. This mutation results in abnormal fibrillin synthesis. Impaired structure of fibrillin protein results in the development of complications within the cardiovascular system, the skeleton and organ of sight. The most common cause of death among patients with Marfan syndrome is aortic disease, but mitral valve dysfunction is also possible. We present the case of a 30- year-old patient with Marfan syndrome who underwent a Bentall de Bono procedure at the age of 18 due to aneurysmal dilation of the aortic bulb. After several years, the patient was qualified again for surgery, because he developed symptomatic severe mitral regurgitation with calcifications present on the mitral annulus and also rupture of the chordae tendineae - which was all confirmed by echocardiography. Due to advanced anatomical changes and the inability to perform plastic surgery on the diseased valve, the patient was implanted with a Sorin Bicarbon 33 mechanical prosthesis. The postoperative course was uneventful. The patient in good general condition was discharged home and was given some recommendations to follow up. The case described emphasizes the importance of long-term cardiological care for patients with Marfan syndrome after aortic surgery. Progressive degenerative changes resulting from defective connective tissue structure may also contribute to the development of pathology in other heart valves. To improve the prognosis in this group of patients, regular echocardiographic monitoring of all heart structures and individualization of treatment strategies are necessary.

Keywords: Marfan syndrome, Bentall de Bono, cardiac surgery, mitral valve

Medical Science, 2025, 29, e215ms3744

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DOI: https://doi.org/10.54905/disssi.v29i165.e215ms3744

Published: 21 November 2025